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难治性铁粒幼细胞性贫血和非铁粒幼细胞性贫血:27例病例回顾

Refractory sideroblastic and nonsideroblastic anemia: a review of 27 cases.

作者信息

Geschke W, Beutler E

出版信息

West J Med. 1977 Aug;127(2):85-92.

Abstract

A study was done with 27 patients who met the following criteria: (1) anemia, (2) cellular bone marrow not diagnostic of leukemia, (3) absence of underlying disease that could account for the hematologic abnormalities at time of initial study and (4) absence of iron, B(12) or folate deficiency. Of the 27 patients, 13 had ringed sideroblasts and 14 did not. Eleven patients received corticosteroids, 18 received folate, 23 pyridoxine and 12 androgens. Two partial responses occurred in the sideroblastic group and were attributed to androgen therapy in one patient and pyridoxine therapy in the other. In the nonsideroblastic group, two partial responses occurred which were attributed to prednisone therapy. Transfusions were required in 23 patients. Leukemia developed in six patients. It is concluded that currently used treatments have little effect on refractory anemia and that in most patients continuing transfusions are required. In a small percentage of patients, there is transformation to leukemia.

摘要

对27例符合以下标准的患者进行了一项研究:(1)贫血;(2)细胞性骨髓未诊断为白血病;(3)在初始研究时不存在可解释血液学异常的基础疾病;(4)不存在铁、维生素B12或叶酸缺乏。27例患者中,13例有环形铁粒幼细胞,14例没有。11例患者接受了皮质类固醇治疗,18例接受了叶酸治疗,23例接受了吡哆醇治疗,12例接受了雄激素治疗。铁粒幼细胞组出现了2例部分缓解,其中1例归因于雄激素治疗,另1例归因于吡哆醇治疗。在非铁粒幼细胞组中,出现了2例部分缓解,归因于泼尼松治疗。23例患者需要输血。6例患者发生了白血病。结论是,目前使用的治疗方法对难治性贫血效果甚微,大多数患者需要持续输血。一小部分患者会转化为白血病。

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本文引用的文献

1
SIDEROBLASTIC ANAEMIA IN MAN: OBSERVATIONS ON SEVENTY CASES.人类铁粒幼细胞性贫血:70例观察报告
Br J Haematol. 1965 Jan;11:59-69. doi: 10.1111/j.1365-2141.1965.tb00085.x.
2
SIDEROBLASTIC ANAEMIA: IS THIS A MALIGNANCY?铁粒幼细胞性贫血:这是一种恶性肿瘤吗?
Br J Haematol. 1965 Jan;11:52-8. doi: 10.1111/j.1365-2141.1965.tb00084.x.
8
Preleukemic acute human leukemia.白血病前期急性人类白血病
J Am Med Assoc. 1953 Jul 11;152(11):1018-28. doi: 10.1001/jama.1953.03690110032010.

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